Journal de néphrologie et thérapeutique

Focal Segmental Glomerulosclerosis Associated Seronegative Antiphospholipid Syndrome

Abstract

Tausif Zar*,Wilner Samson,Ann Parke,M Melinda Sanders,Harold T. Yamase

Secondary Focal Segmental Glomerulosclerosis (FSGS) from thrombotic microangiopathies including Antiphospholipid Antibody Syndrome (APS), is well documented. We present a case with clinical features of APS but consistently negative serologies, suggesting ‘Seronegative APS (SNAPS)’. The patient was evaluated at the Division of Nephrology, University of Connecticut Health Center for progressive Chronic Kidney Disease (CKD). A renal biopsy exhibited thrombotic microangiopathy and associated FSGS.

Systemic thrombophilia can be primary or secondary and has an extensive list of differential diagnoses. Distinct clinical features and serologic markers characterize a particular etiology. Antiphospholipid Syndrome (APS) is the most common acquired thrombophilia. Serologic evidence of APS is the presence of commonly recognized antibodies to phospholipids in this syndrome i.e. anticardiolipin (aCL) antibodies, Lupus Anticoagulant (LA) and β2-glycoprotein 1 (β2GPI) antibodies. Rarely a patient with classic clinical features of APS does not exhibit any of the above antibodies, suggesting ‘Seronegative APS (SNAPS)’.